Malignant peripheral nerve sheath tumours in magnetic resonance imaging : primary and recurrent tumour appearance, post-treatment changes, and metastases

Purpose: To analyse the appearance of primary and recurrent malignant peripheral nerve sheath tumours (MPNSTs) in magnetic resonance imaging (MRI) with a focus on configuration, and to assess the occurrence of loco-regional post-treatment changes and metastases during post-treatment follow-up. Material and methods: Twenty patients with histologically proven MPNST underwent post-treatment 1.5 T MRI. Primary and recurrent MPNSTs were examined for configuration, contrast enhancement, extent, and signal intensity in MRI. Loco-regional post-treatment changes and information on metastases were extracted from the follow-up. Results: MPNSTs occurred most often in the extremities (p = 0.006). Twenty per cent (n = 4) of the patients developed recurrences, with a total of 24 lesions. Recurrent MPNSTs were significantly smaller than primary MPNSTs (p = 0.003). Primary MPNSTs mostly occurred unifocally as multilobulated or ovoid and heterogeneous lesions with mostly well-defined borders. Recurrent MPNSTs purely occurred multifocally as mostly nodular (p < 0.001), multilobulated, or ovoid lesions. 80%, 65% and 30% of the patients showed post-treatment subcutaneous oedema (p = 0.002 to 0.03), muscle oedema (p = 0.02), and seroma, respectively. Twenty-five per cent (n = 5) of patients presented metastases during follow-up. The relative risk in patients with recurrences to develop lung or lymph node metastases is eightfold (p = 0.056). Conclusions: While primary MPNSTs mostly appear unifocally as multilobulated or ovoid lesions, recurrent MPNSTs purely occur multifocally as mostly nodular lesions. Subcutaneous and muscle oedema are very common locoregional post-treatment changes. Patients with recurrences have a higher risk for lung and lymph node metastases

Diagnostic value of MRI for detecting recurrent soft-tissue sarcoma in a long-term analysis at a multidisciplinary sarcoma center

Background Soft-tissue sarcomas (STS) are rare tumors of the soft tissue. Recent diagnostic studies on STS mainly dealt with only few cases of STS and did not investigate the post-therapeutic performance of MRI in a routine clinical setting. Therefore, we assessed the long-term diagnostic accuracy of MRI for detecting recurrent STS at a multidisciplinary sarcoma center. The median age of the patients was 55.3 ± 18.2 years. Of the patients, 34.8% presented with recurrences. Here, 65 follow-up scans were true positive, 23 false positive, 6 false negative, and 961 true negative. The overall sensitivity and specificity of MRI for detecting recurrences were 92 and 98%, respectively, with an accuracy of 97%. For intramuscular lesions and after surgery alone the sensitivity was higher (95 and 97%, respectively) than for subcutaneous lesions and surgery with additional radiation therapy (83 and 86%, respectively), at similarly high specificities (96-98%). The 6 false-negative results were found in streaky (n = 2) and small ovoid/nodular (n = 4) recurring lesions. The false-positive lesions imitated streaky (n = 14), ovoid/nodular (n = 8), and polycyclic/multilobulated recurring tumors (n = 1). All false-positive results were found in patients in whom the primary tumors were polycyclic/multilobulated in appearance. Conclusion MRI shows a high diagnostic accuracy for detecting recurrent STS, with a high sensitivity and specificity. The diagnostic accuracy decreases in subcutaneous lesions and after surgery with radiation therapy, compared to intramuscular lesions and surgery alone. Radiologists should pay particular attention to streaky and small ovoid/nodular recurring lesions and patients with polycyclic/multilobulated primary tumors

Configuration of soft-tissue sarcoma on MRI correlates with grade of malignancy

The aim of the study was to assess whether the configuration of primary soft-tissue sarcoma (STS) on MRI correlates with the grade of malignancy. 71 patients with histologically proven STS were included. Primary STS were examined for configuration, borders, and volume on MRI. The tumors were divided into high-grade (G3), intermediate-grade (G2) and low-grade (G1) STS according to the grading system of the French Federation of Cancer Centers Sarcoma Group (FNCLCC). 30 high-grade, 22 intermediate-grade and 19 low-grade primary STS lesions were identified. High- and intermediate-grade (G3/2) STS significantly most often appeared as polycyclic/multilobulated tumors (p < 0.001 and p = 0.002, respectively). Low-grade (G1) STS mainly showed an ovoid/nodular or streaky configuration (p = 0.008), and well-defined borders. The appearance of high-, intermediate- and low-grade STS with an ovoid/nodular configuration were mainly the same on MRI. All streaky G3/2 sarcoma and 17 of 20 patients with polycyclic/multilobulated G3 sarcoma showed infiltrative borders. High-grade streaky and polycyclic/multilobulated STS are larger in volume, compared to intermediate- and low-grade STS. . Configuration of STS on MRI can indicate the grade of malignancy. Higher-grade (G2/3) STS most often show a polycyclic/multilobulated configuration, while low-grade STS are mainly ovoid/nodular or streaky. Infiltrative behavior might suggest higher-grade STS in streaky and polycyclic/multilobulated STS

Global Retinoblastoma Presentation and Analysis by National Income Level.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs

Global Retinoblastoma Presentation and Analysis by National Income Level

This cross-sectional analysis reports the retinoblastoma stage at diagnosis across the world during a single year, investigates associations between clinical variables and national income level, and investigates risk factors for advanced disease at diagnosis. Key PointsQuestionIs the income level of a country of residence associated with the clinical stage of presentation of patients with retinoblastoma? FindingsIn this cross-sectional analysis that included 4351 patients with newly diagnosed retinoblastoma, approximately half of all new retinoblastoma cases worldwide in 2017, 49.1\% of patients from low-income countries had extraocular tumor at time of diagnosis compared with 1.5\% of patients from high-income countries. MeaningThe clinical stage of presentation of retinoblastoma, which has a major influence on survival, significantly differs among patients from low-income and high-income countries, which may warrant intervention on national and international levels. ImportanceEarly diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. ObjectivesTo report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and ParticipantsA total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and MeasuresAge at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. ResultsThe cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4\%) were female. Most patients (n=3685 {[}84.7\%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n=2638 {[}62.8\%]), followed by strabismus (n=429 {[}10.2\%]) and proptosis (n=309 {[}7.4\%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5\%) patients having intraocular retinoblastoma and 2 (0.3\%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1\%) having extraocular retinoblastoma and 94 of 498 (18.9\%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 {[}95\% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 {[}95\% CI, 4.30-7.68]). Conclusions and RelevanceThis study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs